Adult Congenital Heart Disease

Abstract

It has variably been estimated that between 800 000 and 1 million patients with congenital heart disease are now adults and that by 2010 more patients older than 16 years will undergo surgery for congenital heart disease than those younger than 16 years.1 This review will focus on the clinical aspects of identifying and caring for the portion of the unique population that have right ventricular (RV) outflow tract lesions. The left ventricle (LV) and RV both have inlet, apical, and outlet portions, although they differ considerably.2 The inlet of the RV includes the tricuspid valve, and it is separated from the pulmonary valve (PV) by the crista supraventricularis. This structure results in the PV being raised upward. The apical portion of the RV has coarse trabeculations. As shown in Figure 1, the septum is home to a large septomarginal (septal) trabeculation that divides into the anterior and posterior limbs in the outflow tract. A series of septoparietal trabeculations arise along the free wall. The crista supraventricularis inserts between these limbs and is made up of 3 components: the muscular outlet septum, the ventriculoinfundibular fold, and the inner curvature of the RV.3 The small part that separates the RV and LV cavities is the outlet or conal septum. A loop including the septal trabeculation, the moderator band, septoparietal trabeculations, and the outlet septum can be conceptualized as circling the RV outflow tract. The PV is supported by the circular muscular infundibulum rising outside the RV proper. Abnormalities involving the RV outflow tract involve malpositioning of 1 or more of these structures. Figure 1. The anatomy of the RV outflow tract.2 There is a large septomarginal (septal) trabeculation that divides into 2 limbs. The anterior limb supports the leaflets of the PV, and the posterior limb …